Rosai-Dorfman Disease Presenting as a Pulmonary Artery Mass

Walters, Dustin M.; Dunnington, Gansevoort; Dustin, Simone M.; Frierson, Henry F.; Peeler, Benjamin B.; Kozower, Benjamin D.; Ailawadi, Gorav; Jones, David R.; Lau, Christine L.

doi:10.1016/j.athoracsur.2009.05.016

Cited by 14 publications

(8 citation statements)

References 5 publications

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“…Nevertheless, reports of extranodal RDD involving the carotid sheath and pulmonary artery have been described. [92][93][94] Retroperitoneum, Including Kidneys Histiocytic infiltration of the retroperitoneal space is frequently observed in ECD. By CT, this is seen as a diffuse bilateral infiltrative perinephric soft tissue thickening with a stellate pattern, the so-called hairy kidney, which is highly suggestive of ECD and present in 50% to 68% of patients.…”

Section: Respiratorymentioning

confidence: 99%

The Mayo Clinic Histiocytosis Working Group Consensus Statement for the Diagnosis and Evaluation of Adult Patients With Histiocytic Neoplasms: Erdheim-Chester Disease, Langerhans Cell Histiocytosis, and Rosai-Dorfman Disease

Goyal

Young

Koster

et al. 2019

Mayo Clinic Proceedings

143

160

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Histiocytic neoplasms, a rare and heterogeneous group of disorders, primarily include Erdheim-Chester disease, Langerhans cell histiocytosis, and Rosai-Dorfman disease. Due to their diverse clinical manifestations, the greatest challenge posed by these neoplasms is the establishment of a diagnosis, which often leads to a delay in institution of appropriate therapy. Recent insights into their genomic architecture demonstrating mitogen-activated protein kinase/extracellular signaleregulated kinase pathway mutations have now enabled potential treatment with targeted therapies in most patients. This consensus statement represents a joint document from a multidisciplinary group of physicians at Mayo Clinic who specialize in the management of adult histiocytic neoplasms. It consists of evidence-and consensus-based recommendations on when to suspect these neoplasms and what tests to order for the diagnosis and initial evaluation. In addition, it also describes the histopathologic and individual organ manifestations of these neoplasms to help the clinicians in identifying their key features. With uniform guidelines that aid in identifying these neoplasms, we hope to improve the awareness that may lead to their timely and correct diagnosis.

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Section: Respiratorymentioning

confidence: 99%

The Mayo Clinic Histiocytosis Working Group Consensus Statement for the Diagnosis and Evaluation of Adult Patients With Histiocytic Neoplasms: Erdheim-Chester Disease, Langerhans Cell Histiocytosis, and Rosai-Dorfman Disease

Goyal

Young

Koster

et al. 2019

Mayo Clinic Proceedings

143

160

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“…In a series of nine patients with intrathoracic manifestations of RDD [5], the most common histologic finding was mediastinal lymphadenopathy, followed by interstitial infiltrates, pleural effusion, and cysts. To our knowledge, vasculitis in the setting of RDD has only been reported three times in the literature, once as an intracranial lesion obstructing the carotid artery [6], and twice as lesions involving the aorta [7] and/or main pulmonary artery [7,8]. To date, RDD involving distal pulmonary vessels has not been reported in the English literature.…”

Section: Discussionmentioning

confidence: 99%

Rosai-Dorfman disease of the lung with features of obliterative arteritis

2016

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Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD), is a rare benign non-Langerhans cell histiocytosis. Twenty-five to forty percent of the cases are extranodal and have been reported in virtually all anatomic locations. In this article, we report a highly unusual case of RDD which presented as multiple pulmonary nodules and associated hilar lymphadenopathy. On resection, extensive obliterative arteritis was noticed. This pathologic presentation of RDD has not been reported before in the English literature. Accurate recognition of this entity is crucial to prevent unnecessary aggressive treatment.

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“…Surgical debulking was mandatory because of the lifethreatening localization. [3][4][5] Unilateral pneumonectomy seems more suitable to obtain oncologic radicality in the case of unilateral PA sarcoma, whereas a conservative procedure using pulmonary endarterectomy is indicated in the case of RDD. Therefore, an intraoperative histologic examination is crucial to plan the most appropriate surgical treatment.…”

Section: Discussionmentioning

confidence: 99%

“…Giant dilatation of cardiac atria is usually confined to 1 chamber, very rarely to both, and is mainly related to rheumatic valve diseases of the left heart. [1][2][3] Cases of left atrial enlargement have also been reported with a normally functioning mitral or aortic valve and extremely rarely in the presence of nonrheumatic mitral insufficiency. 2,3 The combination of giant bilateral atrial dilatation and the need of reoperation for cardiac prosthetic dysfunction can pose additional risks because of adhesions and a thin right atrial wall.…”

Section: Discussionmentioning

confidence: 99%